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Purpose of review <p>This review aims to explore how a diagnosis of <i>LMNA</i>-related cardiomyopathy (<i>LMNA</i>-CM) informs clinical management, focusing on the prevention and management of its complications, through practical clinical strategies.</p> Recent findings <p>Longitudinal studies have enhanced our understanding of the natural history of <i>LMNA</i>-CM including its arrhythmic and non-arrhythmic complications. A <i>LMNA</i> specific ventricular arrhythmia risk prediction strategy has been integrated into clinical practice guidelines. Although less robust, observational studies are shaping gene-specific strategies for mitigating other complications including atrioventricular block, atrial fibrillation and cardiomyopathy, while novel therapies have been evaluated in clinical trials.</p> Summary <p><i>LMNA</i>-CM follows an aggressive yet generally stereotyped course. Early recognition of anticipated complications allows for more effective prevention and management in both symptomatic and asymptomatic patients.</p>

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Contemporary Insights into LMNA Cardiomyopathy

  • 期刊论文

  • 发表日期：

    2025年1月27日

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  • Iswaree D. Balakrishnan,
  • Neal K. Lakdawala

摘要

Purpose of review

This review aims to explore how a diagnosis of LMNA-related cardiomyopathy (LMNA-CM) informs clinical management, focusing on the prevention and management of its complications, through practical clinical strategies.

Recent findings

Longitudinal studies have enhanced our understanding of the natural history of LMNA-CM including its arrhythmic and non-arrhythmic complications. A LMNA specific ventricular arrhythmia risk prediction strategy has been integrated into clinical practice guidelines. Although less robust, observational studies are shaping gene-specific strategies for mitigating other complications including atrioventricular block, atrial fibrillation and cardiomyopathy, while novel therapies have been evaluated in clinical trials.

Summary

LMNA-CM follows an aggressive yet generally stereotyped course. Early recognition of anticipated complications allows for more effective prevention and management in both symptomatic and asymptomatic patients.